SHWACHMAN'S SYNDROME Terms of Use. shwachman S syndrome. Specialty Definition shwachman S syndrome. Domain, Definition. Health. Top. Alternative Orthography shwachman S syndrome. http://www.websters-online-dictionary.org/definition/english/Sh/Shwachman's_Synd
Extractions: Philip M. Parker, INSEAD. SHWACHMAN'S SYNDROME Specialty Definition: SHWACHMAN'S SYNDROME Domain Definition A digestive and respiratory disorder of children. Certain digestive enzymes are missing and white blood cells are few. Symptoms may include diarrhea and short stature. ( references Source: compiled by the editor from various references ; see credits. Top Alternative Orthography: SHWACHMAN'S SYNDROME Hexadecimal (or equivalents, 770AD-1900s) references Leonardo da Vinci (1452-1519; backwards) references Binary Code (1918-1938, probably earlier) references HTML Code references ISO 10646 references Encryption (beginner's substitution cypher): references Top google_alternate_ad_url="http://www.websters-online-dictionary.org/js/googlead.asp?kw=SHWACHMAN'S SYNDROME";google_ad_client="pub-7500086874932040"; google_ad_width=728; google_ad_height=90; google_ad_format="728x90_as"; google_color_border="336699"; google_color_bg="FFFFFF";google_color_link="0000FF";google_color_url="008000"; google_color_text="000000"; var rotate_delay = 3000;
Extractions: Information and definitions of the medical conditions and diseases have been taken from various reliable government publications and we have done our best to verify their accuracy. If you feel any of the definitions are incorrect or needs to be updated please contact us and we will look into it. Contact Health Dictionary - Medical and Disease Terms and Definitions
Extractions: Information and definitions of the medical conditions and diseases have been taken from various reliable government publications and we have done our best to verify their accuracy. If you feel any of the definitions are incorrect or needs to be updated please contact us and we will look into it. Contact Health Dictionary - Medical and Disease Terms and Definitions
What's Shwachman's Syndrome? What s shwachman s syndrome? It is a pathological condition, described for the first time by Dr shwachman and by Dr Diamond from http://users.libero.it/aiss/Pag1_ing.html
Extractions: What's Shwachman's syndrome? It is a pathological condition, described for the first time by Dr Shwachman and by Dr Diamond from Boston in 1964, caracterized mainly by ipoplasia of the exocrine pancreas , low height, disorder of medulla ossium and various alterations to the bones. What does it mean? the ipoplasia of the exocrine pancreas indicates a congenital defect about the development of that part of the pancreas that produces the enzymes to digest food. It follows a pancreatic insufficiency and thus an important defect in digestion and food absorption. This defect is inclined to weaken with the years. Low height is present since birth and it is not corrected by any therapy, eventhough the growth has a regular speed. Dysfunctions of medulla ossium are manifold. As a whole there is a scarce development of the medulla, replaced partly by fatty tissue, with consequent scarce production of red corpuscles , blood platelets and the white corpuscles called "granulociti neutrofili", assigned to the defences of front line against the bacteria. The most frequent defect concerns the neutrofili: we talk about neutropenia, that is usually intermittent or cyclic (in other words goes and comes back). Moreover the neutrofili are generally little moving and thus occur with difficulty where there is infection. The scarcity and hypomobility of neutrofili encourage infections, aboove all in a little child: otitis, bronchial pneumonia, osteomyelitis, infections to the cutis, septicaemia.
Magnetic Resonance Imaging For Diagnosis Of Shwachman S Syndrome. Magnetic resonance imaging for diagnosis of shwachman s syndrome. Lacaille F, Mani TM, Brunelle F, Lallemand D, Schmitz J. Department http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=DocSum
Extractions: Initially described in Sweden by R. Kostmann in 1956, infantile genetic agranulocytosis is characterized by severe neutropenia at birth, frequent infections, increased risk of early death, and autosomal-recessive inheritance. This syndrome is one of the severe chronic neutropenic disorders and is mentioned separately only to highlight this well-described clinical entry. The comments regarding treatment and prognosis of severe chronic congenital and idiopathic neutropenias are applicable to Kostmann's neutropenia. Shwachman's Syndrome Intramedullary destruction of neutrophils appears to be the pathophysiologic basis of myelokathexis. Patients demonstrate moderate neutropenia with morphologic abnormality of the neutrophilic nuclei. The circulating neutrophils are notable for their cytoplasmic vacuoles and for very thin nuclear strands connecting the nuclear lobes, while the bone marrow is hyperplastic with many degenerating hypersegmented granulocytes. Cartilage-Hair Hypoplasia Syndrome Short-limbed dwarfism, fine hair, moderate neutropenia, and increased risk of infection characterize cartilage-hair hypoplasia syndrome. This autosomal-recessive disorder is noted most prevalently in the Amish population. Impaired cellular immunity has been noted in some patients. Bone marrow transplantation has been used successfully in at least two patients with this syndrome.
IBMFS - Shwachman-Diamond Syndrome shwachmanDiamond syndrome (SD). SD patients have poor food absorption (malabsorption) and low white blood cell counts (neutropenia). http://www.marrowfailure.cancer.gov/SD.html
Extractions: Other Bone Marrow Failure Syndromes SD patients have poor food absorption (malabsorption) and low white blood cell counts (neutropenia). Most patients are diagnosed in infancy. Males and females are affected equally. What are the major findings on physical examination? Short stature Problems with bones ("metaphyseal dysostosis", a specific finding seen on x-rays). What is the pattern of bone marrow failure? Neutropenia (low white blood count; white cells help the body fight off infection) Anemia (low red blood cell count) may develop (often with large red cells) Low platelet count may develop (platelets are the cells in the blood which help the blood to clot) Aplastic anemia is diagnosed when all 3 types of cells (red cells, white cells and platelets) are abnormally low because the bone marrow is not producing them.
Shwachman Diamond Syndrome shwachman diamond syndrome,. Print this article, shwachman diamond syndrome, Fig. 1. Pelvis and femora of a boy with shwachman Diamond syndome. http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/SHWACHMAN DIAMO
Extractions: Amershamhealth.com Search for: Type a word or a phrase. All forms of the word are searchable. Browse entry words starting with: A B C D ... Other characters Shwachman diamond syndrome, (Harry Shwachman, 20th century, American paediatrician; Louis Diamond, 20th century, American haematologist), (also called metaphyseal chondrodysplasia with exocrine pancreatic insufficiency ), growth retardation, short limb dwarfism, low birth weight, pancreatic insufficiency, malabsorption, recurrent infection and neutropenia. Radiologically, there is mild metaphyseal dysplasia with irregularity of the metaphyses close to the zone of provisional calcification ( Fig.1 ). The pancreas may be shown to be fatty on either MRI or ultrasound.
A To Z Encyclopedia Topic: Shwachman-Diamond Syndrome Clinical Services. shwachmanDiamond syndrome. What is shwachman-Diamond syndrome? shwachman-Diamond syndrome (SDS) is a relatively rare genetic disorder. http://web1.tch.harvard.edu/cfapps/A2ZtopicDisplay.cfm?Topic=Shwachman-Diamond S
Shwachman-Diamond Syndrome INCIDENCE, Rare. GEOGRAPHY, shwachmanDiamond syndrome in a Mexican family. IMMUNE DEFECTS, Immune function in patients with shwachman-Diamond syndrome. http://www.thedoctorsdoctor.com/diseases/shwachman-diamond_syndrome.htm
Extractions: Shwachman-Diamond syndrome in a Mexican family. Belkind-Gerson J, Ontiveros-Nevares P, Ocampo-Roosens V, Sandoval-Juarez D. Departamento de Gastroenterologia, Hospital del Nino Morelense Macaria Than de Rivapalacio, Cuernavaca, Morelos, Mexico. Arch Med Res 2001 Jul-Aug;32(4):318-23 Abstract quote Shwachman-Diamond Syndrome (SDS) is an inherited condition with multisystemic abnormalities including pancreatic exocrine dysfunction, neutropenia, short stature, and skeletal abnormalities. In this report, we describe the case of a 14-year-old female with a history of neutropenia, pancreatic exocrine insufficiency and pancreatic endocrine sufficiency, pancreatic lipomatosis (10), and the development of myeloid leukemia. Postmortem examination revealed a high probability of SDS. We also describe the clinical findings in the patient's six siblings, suggesting this as a familial form of SDS. Because the gene(s) responsible for this syndrome have not yet been identified, genetic confirmation is not yet possible. This is the first report in the literature of a Mexican family with probable SDS.
Shwachman Translate this page shwachman http//shwachman.de/. http://www.shwachman.de/
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